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AH abdominal hysterectomy; absorptive hypercalciuria; accidental hypothermia; acetohexamide; acid hydro...
IH idiopathic hirsutism; idiopathic hypercalciuria; immediate hypersensitivity; incompletely healed; in...
SAHA seborrhea-hypertrichosis/hirsutism-alopecia [syndrome]
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IH Idiopathic Hirsutism
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Apert's hirsutism Hirsutism caused by a virilizing disorder of adrenocortical origin.
(05 Mar 2000)
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Apert Eugene, French paediatrician, 1868-1940.
See: Apert's hirsutism, Apert's syndrome.
(05 Mar 2000)
Apert's syndrome <paediatrics> A usually inherited disorder characterised by premature closing of the cranial suture lines resulting in a peaked shaped head and abnormal facial appearance.
Since it is usually autosomal dominant one or both parents also have the disorder. Surgery is used to correct skull and facial abnormalities.
Inheritance: autosomal dominant.
(29 Dec 1997)
Apert syndrome <syndrome> Apert (1906) defined a syndrome characterised by skull malformation (acrocephaly of brachysphenocephalic type) due to the premature closure of the cranial sutures and syndactyly of the hands and feet of a special type (complete distal fusion with a tendency to fusion also of the bony structures). The hand, when all the fingers are webbed, has been compared to a spoon and, when the thumb is free, to an obstetric hand.
Two clinical categories are distinguished, a 'typical' acrocephalosyndactyly, to which Apert's name is appropriately applied and other forms lumped together as 'atypical' acrocephalosyndactyly.
The feature distinguishing the two types is a middigital hand mass with a single nail common to digits 2-4, found in Apert syndrome and lacking in the others. A frequency of Apert syndrome of 1 in 160,000 births is estimated.
Evidence suggests that Apert syndrome results from mutations in the gene encoding fibroblast growth factor receptor-2.
Progressive synostosis occurs in the feet, hands, carpus, tarsus, cervical vertebrae, and skull, and proposed 'progressive synosteosis with syndactyly' is possibly a more appropriate designation.
Clinical features: flat facies, shallow orbits, hypertelorism, narrow palate, craniosynostosis, brachysphenocephalic acrocephaly, syndactyly, broad thumb, broad great toe, single nail digits 2-4, variable mental retardation, corpus callosum and/or limbic malformations, fused cervical vertebrae.
A skull X-ray can confirm the diagnosis. Treatment is surgical.
Inheritance: autosomal dominant, paternal age effect.
(05 Aug 1998)
constitutional hirsutism Mild to moderate degree of hirsutism present in an individual exhibiting otherwise normal endocrine and reproductive function.
(05 Mar 2000)
hirsutism <clinical sign, dermatology> Abnormal hairiness, especially an adult male pattern of hair distribution in women.
Compare: hypertrichosis.
Origin: Mod. L. Fr. L. Hirsutus, shaggy
(18 Nov 1997)
idiopathic hirsutism Hirsutism of uncertain origin in women, who may additionally exhibit menstrual abnormalities and infertility.
(05 Mar 2000)
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